AB0548 A COMPARATIVE STUDY BETWEEN THE NEUROPSYCHIATRIC INVOLVEMENTS IN ADULT-ONSET AND CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS
Authors: G. K. Yardimci, S. Sener, G. Ayan, A. Taghiyeva, N. P. Acar Ozen, E. D. Batu, L. Kiliç, H. İ. Öncel, A. Tuncer, R. Göçmen, K. Karli Oğuz, Y. Bilginer, U. Kalyoncu, S. Özen, Ş. A. Bilgen
Journal: Annals of the Rheumatic Diseases (2022)

Background Neuropsychiatric(NP) manifestations in systemic lupus erythematosus(SLE) encompass a wide spectrum of neurologic and psychiatric features.The nervous system is frequently affected by adult-onset and childhood-onset SLE patients,but disease course and clinical features may differ between both groups. Objectives The aim of this study is to evaluate and compare NP manifestations in adult-onset and childhood-onset SLE patients. Methods The study included a retrospective analysis of medical records of adult-onset SLE patients and childhood-onset SLE patients following at Hacettepe University from Jan 2015 to Jan 2021.NP events were identified using the ACR established case definitions seen in SLE.Additionally, posterior reversible encephalopathy syndrome, cerebral venous thrombosis, small fiber neuropathy and pseudotumor cerebri were evaluated as having NPSLE in this study.NP were also divided into three categories:neurologic syndromes of the central nervous system, neurologic syndromes of the peripheral nervous system,and diffuse neuropsychological syndromes. Results 1062 adult-onset and 161 childhood-onset SLE patients were followed-up median 150 (84-227) months.At least one neuropsychiatric event occurred in 101 (9.51%) adult-onset and 29 (18.01%) childhood-onset SLE patients (p=0.002). NP events were present before or around the time of SLE diagnosis 64.6% of the adult-onset patients and 72.4% of the childhood-onset patients.One-third of the patients in both groups were diagnosed with NPSLE during the follow-up.At the time of NPSLE diagnosis, 60.4% of adult-onset and 86.2% of childhood-onset patients had additional involvement other than neurologic manifestations and overall disease activity(SLEDAI-2k)was higher in childhood-onset patients (17vs.14p=0.036). Almost all patients in the pediatric group and half of the adult patients were given pulse corticosteroids.Cyclophosphamide was the most preferred regimen and was given to 36.6% of the adult patients and 75.9% of the pediatric patients.Rituximab (11.9% and 27.6%) and mycophenolate mofetil (13.9% and 3.4%) were other induction treatments.The adult-onset and childhood-onset NPSLE patients were followed-up median 105(54-165) and 80(40-132) months, respectively.On the last follow-up visit, disease damage scores (SLICC) were similar in both groups,with a median score of 2. Conclusion The nervous system was more commonly involved in childhood-onset patients, and disease presentations vary significantly between adult-onset and childhood-onset NPSLE patients.Disease activity appeared to be more severe in children at diagnosis, but permanent organ damage was similar in both groups on follow-up. Table 1. Demographics, clinical features of adult-onset and childhood-onset NPSLE patients Adult-onset NPSLE (n=101) Childhood-onset NPSLE (n=29) P value Cerebrovascular disease 63 (62.4) 8 (27.6) 0.001 Arterial cerebrovascular events 44 (43.6) 5 (17.2) 0.007 Central nervous system vasculitis 25 (24.8) 10 (34.5) 0.209 Parenchymal involvement 18 (17.8) 3 (10.3) 0.256 Demyelinating syndrome 14 (13.9) 2 (6.9) 0.221 Transient ischemic attack 5 (5.0) - 0.277 Cerebral venous sinus thrombosis 4 (4.0) 6 (20.7) 0.008 Meningeal involvement 5 (5.0) - 0.277 Posterior reversible encephalopathy syndrome 3 (3.0) 2 (6.9) 0.310 Pseudotumor cerebri 7 (6.9) 3 (10.3) 0.393 Idiopathic intracranial hypertension 2 (2.0) 3 (10.3) 0.073 Lupus headache 38 (37.6) 18 (62.1) 0.017 Acute confusional state 13 (12.9) 4 (13.8) 0.555 Cognitive dysfunction 9 (8.9) 1 (3.4) 0.299 Seizure disorders 31 (30.7) 11 (37.9) 0.302 Movement disorder (chorea) 4 (4.0) 2 (6.9) 0.402 Myasthenia gravis 3 (3.0) - 0.466 Cranial neuropathy 7 (6.9) 3 (10.3) 0.393 Optic neuritis 4 (4.0) 1 (3.4) 0.690 Peripheral polyneuropathy 9 (8.9) 5 (17.2) 0.173 Mononeuritis multiplex 1 (1.0) - 0.777 Myelopathy 5 (5.0) 2 (6.9) 0.489 Autonomic dysfunction - - - Small fiber neuropathy 1 (1.0) - 0.777 Figure 1. Distribution of adult-onset and childhood-onset NPSLE patients Disclosure of Interests None declared